11β-hydroxylase deficiency medical therapy: Difference between revisions

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{{Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency}}
{{11β-hydroxylase deficiency}}
{{CMG}}; {{AE}} {{MJ}}
{{CMG}}; {{AE}} {{MJ}}
==Overview==
==Overview==
Treatment for 11beta-hydroxylase deficiency in children is glucocorticoids such as hydrocortisone. The response to therapy should be monitored by laboratory tests and clinical findings. Glucocorticoid-related side effects such as bruising, weight gain, and glucose intolerance should be monitored. Genital malformations in females may require surgical correction with one or more surgeries and vaginal dilation. In adult women with 11beta-hydroxylase deficiency androgen excess and hypertension remain indications for treatment. The treatment option in this groups is spironolactone.If pregnancy is not desired, spironolactone plus an oral contraception pill can be combined with replacement doses of hydrocortisone.In adult males, replacement doses of hydrocortisone should be administered to avoid the development of adrenal rest tumors.
Treatment for 11β-hydroxylase deficiency in children is administration of [[glucocorticoids]]. The response to therapy should be monitored by laboratory tests and clinical findings. The treatment option in women is spironolactone. If pregnancy is not desired, [[spironolactone]] plus [[oral contraceptive pills]] can be combined with replacement doses of [[hydrocortisone]]. In adult males, replacement doses of [[hydrocortisone]] should be administered to avoid the development of [[Adrenal tumor|adrenal rest tumors]].


==Medical Therapy==
==Medical Therapy==
* Treatment for 11beta-hydroxylase deficiency in children is glucocorticoids such as:
* Treatment for 11β-hydroxylase deficiency in children is [[glucocorticoids]] as followings:
** Including hydrocortisone (10 to 25 mg/m2)
** Preferred regimen (1): [[Hydrocortisone]] 10 to 25 mg/m2 [[body surface area]]/day PO
** Prednisolone (0.1 mg/kg)
** Preferred regimen (2): [[Hydrocortisone|Prednisolone]]  0.1 mg/kg/day PO
** Dexamethasone (up to 0.5 mg/day).
** Preferred regimen (3): [[Dexamethasone]] up to 0.5 mg/day PO


* The response to therapy should be monitored by:
* The response to therapy should be monitored by:
** Laboratory tests such as serum DHEAS, potassium levels, and 11-deoxycortisol.
** Laboratory tests such as serum [[DHEAS]], [[potassium]] levels, and [[Deoxycortisol|11-deoxycortisol]].
** Clinical findings such virilization, growth velocity, and skeletal maturation (bone age)
** Clinical findings such [[virilization]], [[growth]] velocity, and [[bone age]].
* Glucocorticoid-related side effects such as bruising, weight gain, and glucose intolerance should be monitored.  
* [[Glucocorticoid]]-related side effects such as [[bruising]], [[weight gain]], and [[glucose intolerance]] should be monitored.  


* Genital malformations in females may require surgical correction with one or more surgeries and vaginal dilation.  
* [[Genital]] malformations in females may require surgical correction with one or more surgeries and vaginal dilation.  


* In adult women with 11beta-hydroxylase deficiency androgen excess and hypertension remain indications for treatment. The treatment option in this groups:
* In women with 11β-hydroxylase deficiency, [[androgen]] excess and [[hypertension]] are indications for treatment. The treatment option in this groups:
** Spironolactone, 25 to 200 mg/day
** Preferred regimen (1): [[Spironolactone]] 25 to 200 mg/day PO
** If pregnancy is not desired, spironolactone plus an oral contraception pill can be combined with replacement doses of hydrocortisone.
** If [[pregnancy]] is not desired, [[oral contraceptive pills]] can be combined with [[hydrocortisone]].


* In adult males, replacement doses of hydrocortisone should be administered to avoid the development of adrenal rest tumors.<ref name="pmid28576284">{{cite journal |vauthors=El-Maouche D, Arlt W, Merke DP |title=Congenital adrenal hyperplasia |journal=Lancet |volume= |issue= |pages= |year=2017 |pmid=28576284 |doi=10.1016/S0140-6736(17)31431-9 |url=}}</ref>
* In adult males, replacement doses of [[hydrocortisone]] should be administered to avoid the development of [[Adrenal tumor|adrenal rest tumors]].<ref name="pmid28576284">{{cite journal |vauthors=El-Maouche D, Arlt W, Merke DP |title=Congenital adrenal hyperplasia |journal=Lancet |volume= |issue= |pages= |year=2017 |pmid=28576284 |doi=10.1016/S0140-6736(17)31431-9 |url=}}</ref>


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}

Latest revision as of 16:53, 3 August 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]

Overview

Treatment for 11β-hydroxylase deficiency in children is administration of glucocorticoids. The response to therapy should be monitored by laboratory tests and clinical findings. The treatment option in women is spironolactone. If pregnancy is not desired, spironolactone plus oral contraceptive pills can be combined with replacement doses of hydrocortisone. In adult males, replacement doses of hydrocortisone should be administered to avoid the development of adrenal rest tumors.

Medical Therapy

  • Genital malformations in females may require surgical correction with one or more surgeries and vaginal dilation.

References

  1. El-Maouche D, Arlt W, Merke DP (2017). "Congenital adrenal hyperplasia". Lancet. doi:10.1016/S0140-6736(17)31431-9. PMID 28576284.